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Hypoplastic left heart syndrome

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https://www.readbyqxmd.com/read/28534240/an-early-glenn-operation-may-be-associated-with-the-later-occurrence-of-protein-losing-enteropathy-in-fontan-patients-association-of-early-glenn-and-failing-fontan
#1
Bettina Unseld, Brigitte Stiller, Thomas Borth-Bruhns, Florian du Bois, Johannes Kroll, Jochen Grohmann, Thilo Fleck
Protein-losing enteropathy (PLE) and plastic bronchitis (PB) are major causes of long-term mortality after Fontan operation. The objective of this study was to determine early clinical risk factors before the onset of PLE and PB. In a cohort study, 106 Fontan patients between 2005 and 2013 were examined. A median of 5.3 (1.5-8.5) years later, follow-up questionnaires were used to group the patients in a PLE or PB group (n = 14) and a non-PLE/PB group (n = 92). Prevalence of PLE was 9.4% (n = 10) and of PB 3...
May 22, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28530678/the-complex-genetics-of-hypoplastic-left-heart-syndrome
#2
Xiaoqin Liu, Hisato Yagi, Shazina Saeed, Abha S Bais, George C Gabriel, Zhaohan Chen, Kevin A Peterson, You Li, Molly C Schwartz, William T Reynolds, Manush Saydmohammed, Brian Gibbs, Yijen Wu, William Devine, Bishwanath Chatterjee, Nikolai T Klena, Dennis Kostka, Karen L de Mesy Bentley, Madhavi K Ganapathiraju, Phillip Dexheimer, Linda Leatherbury, Omar Khalifa, Anchit Bhagat, Maliha Zahid, William Pu, Simon Watkins, Paul Grossfeld, Stephen A Murray, George A Porter, Michael Tsang, Lisa J Martin, D Woodrow Benson, Bruce J Aronow, Cecilia W Lo
Congenital heart disease (CHD) affects up to 1% of live births. Although a genetic etiology is indicated by an increased recurrence risk, sporadic occurrence suggests that CHD genetics is complex. Here, we show that hypoplastic left heart syndrome (HLHS), a severe CHD, is multigenic and genetically heterogeneous. Using mouse forward genetics, we report what is, to our knowledge, the first isolation of HLHS mutant mice and identification of genes causing HLHS. Mutations from seven HLHS mouse lines showed multigenic enrichment in ten human chromosome regions linked to HLHS...
May 22, 2017: Nature Genetics
https://www.readbyqxmd.com/read/28526502/hemodynamic-parameters-predict-adverse-outcomes-following-biventricular-conversion-with-single-ventricle-palliation-takedown
#3
Melissa A Herrin, David Zurakowski, Christopher W Baird, Puja Banka, Jesse J Esch, Pedro J Del Nido, Sitaram M Emani
OBJECTIVE: Patients with a borderline left ventricular hypoplasia in the hypoplastic left heart syndrome variant or an unbalanced atrioventricular canal who undergo initial single-ventricle palliation may be candidates for biventricular (BiV) conversion following left ventricle (LV) recruitment procedures. We investigated associations among preoperative parameters and postoperative outcomes in patients undergoing BiV conversion. METHODS: We performed a retrospective review of patients who underwent BiV conversion to determine variables associated with clinical outcomes...
April 11, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28521042/induced-pluripotent-stem-cell-modelling-of-hlhs-underlines-the-contribution-of-dysfunctional-notch-signalling-to-impaired-cardiogenesis
#4
Chunbo Yang, Yaobo Xu, Min Yu, David Lee, Sameer Alharti, Nicola Hellen, Noor Ahmad Shaik, Babajan Banaganapalli, Hussein Ali Mohamoud Sheikh, Elango Ramu, Stefan Przyborski, Gennadiy Tenin, Simon Williams, John O'Sullivan, Osman O Al-Radi, Jameel Atta, Sian E Harding, Bernard Keavney, Majlinda Lako, Lyle Armstrong
Hypoplastic left heart syndrome (HLHS) is among the most severe forms of congenital heart disease. Although the consensus view is that reduced flow through the left heart during development is a key factor in the development of the condition, the molecular mechanisms leading to hypoplasia of left heart structures are unknown. We have generated induced pluripotent stem cells (iPSC) from five HLHS patients and two unaffected controls, differentiated these to cardiomyocytes and identified reproducible in vitro cellular and functional correlates of the HLHS phenotype...
May 17, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28512719/hypoplastic-left-heart-syndrome-with-congenital-complete-heart-block
#5
Maytham Al-Kubaisi, Safwat A Aly, Bassel Mohammad Nijres, Sawsan Awad
Congenital complete atrioventricular block (CCAVB) is a rare condition with an incidence of 1 of 20,000 live births. Hypoplastic left heart syndrome (HLHS) occurs more frequently than CCAVB and occurs in 1 of 5000 live births. HLHS in association with CCAVB is exceedingly rare. In this report, we describe a rare case of HLHS and CCAVB diagnosed in utero. Postnatal diagnosis, management and outcome are presented as well as review of the medical literature.
May 17, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28508919/cognitive-development-of-school-age-hypoplastic-left-heart-syndrome-survivors-a-single-center-study
#6
REVIEW
Raphael D Oberhuber, Sonja Huemer, Rudolf Mair, Eva Sames-Dolzer, Michaela Kreuzer, Gerald Tulzer
Neurological and radiologic research results show an abnormal cerebral microstructure as well as abnormal neurodevelopment in patients treated for hypoplastic left heart syndrome. The aim of this study was to assess the varying cognitive performance these children have developed in dependence upon prenatal diagnosis, surgical techniques, surgical learning effects, anatomy, perfusion techniques, gender, pedagogic, and sociodemographic parameters in comparison to age-adjusted normative values. School-age children (6...
May 16, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28491863/reproducing-patient-specific-hemodynamics-in-the-blalock-taussig-circulation-using-a-flexible-multi-domain-simulation-framework-applications-for-optimal-shunt-design
#7
Christopher J Arthurs, Pradyumn Agarwal, Anna V John, Adam L Dorfman, Ronald G Grifka, C Alberto Figueroa
For babies born with hypoplastic left heart syndrome, several open-heart surgeries are required. During Stage I, a Norwood procedure is performed to construct an appropriate circulation to both the systemic and the pulmonary arteries. The pulmonary arteries receive flow from the systemic circulation, often using a Blalock-Taussig (BT) shunt between the innominate artery and the right pulmonary artery. This procedure causes significantly disturbed flow in the pulmonary arteries. In this study, we use computational hemodynamic simulations to demonstrate its capacity for examining the properties of the flow through and near the BT shunt...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28471442/an-antenatal-marker-of-neurodevelopmental-outcomes-in-infants-with-congenital-heart-disease
#8
S Siddiqui, W P Fifer, M Ordonez-Retamar, J D Nugent, I A Williams
OBJECTIVE: Prenatal exposures are known to alter fetal neurodevelopment and autonomic control. We aimed to explore the correlation between fetal autonomic activity, measured by fetal heart rate variability, and 18-month developmental outcome in subjects with congenital heart disease. STUDY DESIGN: From 2010 to 2013, 5 fetuses with hypoplastic left heart syndrome, 9 with transposition of the great arteries and 9 with tetralogy of Fallot were included in this prospective cohort study...
May 4, 2017: Journal of Perinatology: Official Journal of the California Perinatal Association
https://www.readbyqxmd.com/read/28456482/hospital-costs-and-cost-implications-of-co-morbid-conditions-for-patients-with-single-ventricle-in-the-period-through-to-fontan-completion
#9
Li Huang, Kim M Dalziel, Chris Schilling, David S Celermajer, John J McNeil, David Winlaw, Tom Gentles, Dorothy J Radford, Michael Cheung, Andrew Bullock, Gavin R Wheaton, Robert N Justo, Lisa A Selbie, Victoria Forsdick, Karin Du Plessis, Yves d'Udekem
BACKGROUND: Patients undergoing palliative surgeries for single-ventricle conditions are affected by multiple comorbidities or non-cardiac conditions. The prevalence, costs and the cost implications of these conditions have not been assessed. METHODS: Administrative costing records from four hospitals in Australia and New Zealand were linked with the Fontan registry database to analyze the inpatient resource use for co-morbid or non-cardiac conditions. Inpatient costing records from the birth year through to Fontan completion were available for 156 patients...
April 20, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28446969/micrornas-pleiotropic-players-in-congenital-heart-disease-and-regeneration
#10
REVIEW
Sarah C Hoelscher, Stefanie A Doppler, Martina Dreßen, Harald Lahm, Rüdiger Lange, Markus Krane
Congenital heart disease (CHD) is the leading cause of infant death, affecting approximately 4-14 live births per 1,000. Although surgical techniques and interventions have improved significantly, a large number of infants still face poor clinical outcomes. MicroRNAs (miRs) are known to coordinately regulate cardiac development and stimulate pathological processes in the heart, including fibrosis or hypertrophy and impair angiogenesis. Dysregulation of these regulators could therefore contribute (I) to the initial development of CHD and (II) at least partially to the observed clinical outcomes of many CHD patients by stimulating the aforementioned pathways...
March 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28444210/initial-shunt-type-at-the-norwood-operation-impacts-myocardial-function-in-hypoplastic-left-heart-syndrome%C3%A2
#11
Hanna K Ruotsalainen, Jaana Pihkala, Jukka Salminen, Lisa K Hornberger, Heikki Sairanen, Tiina Ojala
OBJECTIVES: We investigated the impact of initial shunt type, a Blalock-Taussig (BT) shunt versus a right ventricle to pulmonary artery conduit (RV-PA) on myocardial function at different stages of surgical palliation in patients with hypoplastic left heart syndrome (HLHS). METHODS: A population-based cohort of 63 Finnish children with HLHS (BT n  = 23, RV-PA n  = 40) born between 2003 and 2010 were studied retrospectively by echocardiography prior to Stages 1, 2 and 3 palliation and 0...
April 24, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28410632/evolution-of-left-ventricular-size-in-late-survivors-of-surgery-for-hypoplastic-left-heart-syndrome
#12
Markus Bjurbom, Ajay J Iyengar, Florian Moenkemeyer, Igor E Konstantinov, Christian P Brizard, Yves d'Udekem
BACKGROUND: Aortic atresia and mitral atresia are predictors of adverse events in early life in patients with hypoplastic left heart syndrome, but it is unclear whether late outcomes are also affected, and whether this impact is related to a small hypertrophied left ventricle (LV) that impairs right ventricle (RV) function. METHODS: Thirty patients with hypoplastic left heart syndrome surviving with a Fontan procedure were identified. Follow-up echocardiograms were available at birth, before the Fontan procedure, and several years after...
April 12, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28408455/cell-therapy-trials-in-congenital-heart-disease
#13
REVIEW
Hidemasa Oh
Dramatic evolution in medical and catheter interventions and complex surgeries to treat children with congenital heart disease (CHD) has led to a growing number of patients with a multitude of long-term complications associated with morbidity and mortality. Heart failure in patients with hypoplastic left heart syndrome predicated by functional single ventricle lesions is associated with an increase in CHD prevalence and remains a significant challenge. Pathophysiological mechanisms contributing to the progression of CHD, including single ventricle lesions and dilated cardiomyopathy, and adult heart disease may inevitably differ...
April 14, 2017: Circulation Research
https://www.readbyqxmd.com/read/28402400/unplanned-cardiac-reoperations-and-interventions-during-long-term-follow-up-after-the-norwood-procedure%C3%A2
#14
Eva Sames-Dolzer, Gregor Gierlinger, Michaela Kreuzer, Julia Schrempf, Roland Gitter, Christoph Prandstetter, Gerald Tulzer, Rudolf Mair
OBJECTIVES: Patients with hypoplastic left heart syndrome or related malformations are predominantly treated with a 3-stage palliation. Anatomic or physiologic problems can lead to unplanned additional surgical or catheter interventions during single ventricle palliation. Changes in operative technique may have an impact on the reoperation rate. METHODS: Between 1997 and 2014, 317 Norwood procedures were performed at our centre. A retrospective single centre investigation was carried out concerning incidence, timing, indication and type of unplanned interstage cardiac reoperations and catheter interventions during follow-up of Norwood patients...
June 1, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28393581/the-effect-of-transport-on-the-physiologic-stability-of-neonates-withductal-dependent-single-ventricle-lesions
#15
Silvestre R Duran, Sanjeev Aggarwal, Girija Natarajan
OBJECTIVE: To compare the status of infants with hypoplastic left heart syndrome (HLHS) or pulmonary atresia-hypoplastic right heart (PA-HRH) before and following transport using the validated Transport Risk Index of Physiologic Stability (TRIPS) score. METHODS: In this retrospective review of infants with HLHS or PA-HRH transported to a Children's Hospital by a pediatric transport team, an increase in TRIPS score (temperature, blood pressure, respiratory status, and response to stimuli) following transport was defined as deterioration...
April 10, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28371265/oral-facial-digital-syndrome-type-1-in-males-congenital-heart-defects-are-included-in-its-phenotypic-spectrum
#16
Arjan Bouman, Mariëlle Alders, Roelof Jan Oostra, Elisabeth van Leeuwen, Nikki Thuijs, Anne-Marie van der Kevie-Kersemaekers, Merel van Maarle
Oral-facial-digital syndrome type 1 (OFD1; OMIM# 311200) is an X-linked dominant ciliopathy caused by mutations in the OFD1 gene. This condition is characterized by facial anomalies and abnormalities of oral tissues, digits, brain, and kidneys. Almost all affected patients are female, as OFD1 is presumed to be lethal in males, mostly in the first or second trimester of pregnancy. Live born males with OFD1 are a rare occurrence, with only five reported patients to date. In four patients the presence of a congenital heart defect (CHD) was observed...
May 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28365652/interstage-home-monitoring-after-newborn-first-stage-palliation-for-hypoplastic-left-heart-syndrome-family-education-strategies
#17
Jo Ann Nieves, Karen Uzark, Nancy A Rudd, Jennifer Strawn, Anne Schmelzer, Nancy Dobrolet
Children born with hypoplastic left heart syndrome are at high risk for serious morbidity, growth failure, and mortality during the interstage period, which is the time from discharge home after first-stage hypoplastic left heart syndrome palliation until the second-stage surgical intervention. The single-ventricle circulatory physiology is complex, fragile, and potentially unstable. Multicenter initiatives have been successfully implemented to improve outcomes and optimize growth and survival during the interstage period...
April 2017: Critical Care Nurse
https://www.readbyqxmd.com/read/28359493/twin-to-twin-heart-transplantation-a-unique-event-with-a-25-year-follow-up
#18
David Blitzer, Grace Yedlicka, Joshua Manghelli, John Dentel, Randall Caldwell, John W Brown
Solid organ transplantation in pediatric patients has been a reality since 1954, when the first kidney transplantation was successfully performed between identical twins. We report the long-term outcomes, with more than 25 years of follow-up, in a patient born with hypoplastic left heart syndrome (HLHS) who received a heart transplant from a dizygotic twin. While we would not wish for this situation to reoccur, we hope that in reporting it, we can add to the discussion surrounding pediatric heart transplantation and the management of HLHS...
April 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28345115/counseling-practices-for-fetal-hypoplastic-left-heart-syndrome
#19
Michael J Walsh, George R Verghese, M Eric Ferguson, Nora F Fino, David J Goldberg, Sonal T Owens, Nelangi Pinto, Sinai C Zyblewski, Michael D Quartermain
While counseling parents of a fetus diagnosed with hypoplastic left heart syndrome (HLHS), pediatric cardiologists play a critical role in shaping a family's expectations for the months and years to come. However, techniques for the most effective counseling practices have not been studied, and significant variation among physicians is likely present. Web-based survey of pediatric cardiologists that perform fetal echocardiography using snowball sampling. 201 physicians responded (61% male, 81% from academic centers, and 95% from the U...
March 27, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28341901/hypoplastic-left-heart-syndrome-is-not-associated-with-worse-clinical-or-neurodevelopmental-outcomes-than-other-cardiac-pathologies-after-the-norwood-sano-operation
#20
Billie-Jean Martin, I De Villiers Jonker, Ari R Joffe, Gwen Y Bond, Bryan V Acton, David B Ross, Charlene M T Robertson, Ivan M Rebeyka, Joseph Atallah
There is evidence to suggest that patients undergoing a Norwood for non-HLHS anatomy may have lower mortality than classic HLHS, but differences in neurodevelopmental outcome have not been assessed. Our objective was to compare survival and neurodevelopmental outcome during the same surgical era in a large, well-described cohort. All subjects who underwent a Norwood-Sano operation between 2005 and 2014 were included. Follow-up clinical, neurological, and developmental data were obtained from the Western Canadian Complex Pediatric Therapies Follow-up Program database...
March 24, 2017: Pediatric Cardiology
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